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Subacute Vision Loss in a Patient With Limited Immune Dysfunction

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 62-year-old man presented with subacute, painless vision loss in the right eye that developed over the course of 1 week. He had a history of well-controlled type 2 diabetes and diffuse large B-cell lymphoma that was diagnosed 3 years earlier. The lymphoma was initially treated with chemotherapy, followed by a relapse that required additional chemotherapy and ultimately autologous bone marrow transplant 3 months before presentation. At the time of presentation, he was taking no immunosuppressive medications and was taking prophylactic acyclovir, 800 mg twice daily, and sulfamethoxazole/trimethoprim (800 mg/1600 mg) 3 times weekly.

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Chronic retinal necrosis due to cytomegalovirus

C. Administer intravitreal foscarnet and initiate valganciclovir

Based on the constellation of clinical findings, there was high clinical suspicion for cytomegalovirus (CMV) retinitis, so the patient was treated empirically with intravitreal foscarnet, 2.4 mg, and oral valganciclovir, 900 mg, twice daily. Aqueous tap and polymerase chain reaction analysis results returned 3.5 million copies of CMV DNA, confirming a diagnosis of chronic retinal necrosis (CRN) due to CMV.

Cytomegalovirus retinitis typically occurs in patients with severe immunosuppression and has been well described in patients with AIDS.1,2 Cytomegalovirus retinitis often lacks substantial intraocular inflammation and may manifest as either an indolent progressive peripheral granular necrotizing retinitis or as a fulminant posterior hemorrhagic retinitis.1,2

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Article Information

Corresponding Author: Mark W. Johnson, MD, University of Michigan, Kellogg Eye Center, 1000 Wall St, Ann Arbor, MI 48105 (markwj@med.umich.edu).

Published Online: December 12, 2019. doi:10.1001/jamaophthalmol.2019.4915

Conflict of Interest Disclosures: Dr Wubben reported consulting fees from Allergan outside the submitted work. Dr Johnson reported serving on data safety monitoring boards for Pfizer and Syneos Health. No other disclosures were reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Holland  GN, Vaudaux  JD, Jeng  SM,  et al; UCLA CMV Retinitis Study Group.  Characteristics of untreated AIDS-related cytomegalovirus retinitis; I: findings before the era of highly active antiretroviral therapy (1988 to 1994).  Am J Ophthalmol. 2008;145(1):5-11. doi:10.1016/j.ajo.2007.09.023PubMedGoogle ScholarCrossref
2.
Holland  GN, Vaudaux  JD, Shiramizu  KM,  et al; Southern California HIV/Eye Consortium.  Characteristics of untreated AIDS-related cytomegalovirus retinitis, II: findings in the era of highly active antiretroviral therapy (1997 to 2000).  Am J Ophthalmol. 2008;145(1):12-22. doi:10.1016/j.ajo.2007.09.040PubMedGoogle ScholarCrossref
3.
Holland  GN, Van Natta  ML, Goldenberg  DT, Ritts  R  Jr, Danis  RP, Jabs  DA; Studies of Ocular Complications of AIDS Research Group.  Relationship between opacity of cytomegalovirus retinitis lesion borders and severity of immunodeficiency among people with AIDS.  Invest Ophthalmol Vis Sci. 2019;60(6):1853-1862. doi:10.1167/iovs.18-26517PubMedGoogle ScholarCrossref
4.
Schneider  EW, Elner  SG, van Kuijk  FJ,  et al.  Chronic retinal necrosis: cytomegalovirus necrotizing retinitis associated with panretinal vasculopathy in non-HIV patients.  Retina. 2013;33(9):1791-1799. doi:10.1097/IAE.0b013e318285f486PubMedGoogle ScholarCrossref
5.
Schoenberger  SD, Kim  SJ, Thorne  JE,  et al.  Diagnosis and treatment of acute retinal necrosis: a report by the American Academy of Ophthalmology.  Ophthalmology. 2017;124(3):382-392. doi:10.1016/j.ophtha.2016.11.007PubMedGoogle ScholarCrossref
6.
Goldberg  DE, Smithen  LM, Angelilli  A, Freeman  WR.  HIV-associated retinopathy in the HAART era.  Retina. 2005;25(5):633-649. doi:10.1097/00006982-200507000-00015PubMedGoogle ScholarCrossref
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Taplitz  RA, Kennedy  EB, Bow  EJ,  et al.  Antimicrobial prophylaxis for adult patients with cancer-related immunosuppression: ASCO and IDSA clinical practice guideline update.  J Clin Oncol. 2018;36(30):3043-3054. doi:10.1200/JCO.18.00374PubMedGoogle ScholarCrossref
8.
Tomblyn  M, Chiller  T, Einsele  H,  et al.  Guidelines for preventing infectious complications among hematopoietic cell transplant recipients: a global perspective.  Bone Marrow Transplant. 2009;44(8):453-455. doi:10.1038/bmt.2009.254Google ScholarCrossref
9.
Kaplan  JE, Benson  C, Holmes  KK, Brooks  JT, Pau  A, Masur  H; Centers for Disease Control and Prevention (CDC); National Institutes of Health; HIV Medicine Association of the Infectious Diseases Society of America.  Guidelines for prevention and treatment of opportunistic infections in HIV-infected adults and adolescents: recommendations from CDC, the National Institutes of Health, and the HIV Medicine Association of the Infectious Diseases Society of America.  MMWR Recomm Rep. 2009;58(RR-4):1-207.PubMedGoogle Scholar
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